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膀胱副神经节瘤:生物学潜能能否被预测?

Paraganglioma of the urinary bladder: can biologic potential be predicted?

作者信息

Cheng L, Leibovich B C, Cheville J C, Ramnani D M, Sebo T J, Neumann R M, Nascimento A G, Zincke H, Bostwick D G

机构信息

Department of Pathology, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA.

出版信息

Cancer. 2000 Feb 15;88(4):844-52. doi: 10.1002/(sici)1097-0142(20000215)88:4<844::aid-cncr15>3.0.co;2-i.

DOI:10.1002/(sici)1097-0142(20000215)88:4<844::aid-cncr15>3.0.co;2-i
PMID:10679654
Abstract

BACKGROUND

Paraganglioma of the urinary bladder is rarely encountered and its biologic behavior is uncertain. The authors sought to determine the prognostic factors that would predict patient outcome.

METHODS

The Mayo Clinic experience over 53 years with paraganglioma of the bladder was reviewed. All histologic slides from 16 patients were reviewed by the authors. Eight cases were examined immunohistochemically with cytokeratin (AE1/3, cytokeratin 7, and cytokeratin 20), vimentin, S-100 protein, neuroendocrine markers (chromogranin, synaptophysin, and neuron specific enolase), p53 protein, and MIB-1. DNA ploidy was determined by digital image analysis in formalin fixed, paraffin embedded tissue. The mean follow-up was 6.3 years (range, 0.4-16.4 years).

RESULTS

Paraganglioma usually occurred in young adult women (mean age, 45 years; range, 16-74 years). The male-to-female ratio was 1 to 3. The common symptoms and signs were hypertension and hematuria. The tumors were usually located intramurally in the lateral and posterior wall of the bladder and were multifocal in 3 cases (18%). Seven patients were treated by transurethral resection, eight by partial cystectomy, and one by radical cystectomy. T classification was T1 (1 patient), T2 (9 patients), T3 (2 patients), and T4b (4 patients). At the time of diagnosis, one patient had distant metastasis and one had regional lymph node metastasis. One patient developed metastasis 1 year after diagnosis and died of the disease 1.5 years later. None of the patients with T1 or T2 tumors had recurrence or tumor progression. All tumors were aneuploid. The mean MIB-1 labeling index was 1.5% (range, 0.03-7.0%). The tumor cells displayed immunoreactivity for S-100 protein and neuroendocrine markers and were negative for p53 (except 1 case) and cytokeratin.

CONCLUSIONS

Paraganglioma of the urinary bladder occurs mostly in young adult women. Patients with tumor of advanced classification (>/=T3) are at risk of recurrence, metastasis, and dying of the disease, whereas patients in this study with T1 or T2 disease had favorable outcomes after complete tumor resection.

摘要

背景

膀胱副神经节瘤罕见,其生物学行为尚不确定。作者试图确定可预测患者预后的预后因素。

方法

回顾了梅奥诊所53年来膀胱副神经节瘤的诊治经验。作者复查了16例患者的所有组织学切片。8例进行了免疫组织化学检查,检测细胞角蛋白(AE1/3、细胞角蛋白7和细胞角蛋白20)、波形蛋白、S-100蛋白、神经内分泌标志物(嗜铬粒蛋白、突触素和神经元特异性烯醇化酶)、p53蛋白和MIB-1。通过数字图像分析在福尔马林固定、石蜡包埋组织中测定DNA倍体。平均随访时间为6.3年(范围0.4 - 16.4年)。

结果

膀胱副神经节瘤通常发生于年轻成年女性(平均年龄45岁;范围16 - 74岁)。男女比例为1比3。常见症状和体征为高血压和血尿。肿瘤通常位于膀胱壁内、侧壁和后壁,3例(18%)为多灶性。7例患者接受经尿道切除术,8例接受部分膀胱切除术,1例接受根治性膀胱切除术。T分期为T1(1例患者)、T2(9例患者)、T3(2例患者)和T4b(4例患者)。诊断时,1例患者有远处转移,1例有区域淋巴结转移。1例患者诊断后1年发生转移,1.5年后死于该病。T1或T2期肿瘤患者均无复发或肿瘤进展。所有肿瘤均为非整倍体。平均MIB-1标记指数为1.5%(范围0.03 - 7.0%)。肿瘤细胞对S-100蛋白和神经内分泌标志物呈免疫反应性,对p53(1例除外)和细胞角蛋白呈阴性。

结论

膀胱副神经节瘤主要发生于年轻成年女性。高级别分类(≥T3)肿瘤患者有复发、转移和死于该病的风险,而本研究中T1或T2期疾病患者在肿瘤完全切除后预后良好。

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